CLN6 (N258) polyclonal Antibody | BS3102

CLN6 (N258) polyclonal Antibody | BS3102 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse,Rat

Application: WB IHC

Application Range: WB: 1:500~1:1000 IHC: 1:50~1:200

Background: CLN6, a 311-amino acid protein, has 7 predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness; part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: CLN6 (N258) polyclonal Antibody detects endogenous levels of CLN6 protein.

Molecular Weight: ~ 40 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6; CLN6

Immunogen: Synthetic peptide, corresponding to amino acids 229-274 of Human CLN6.

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Pathway: